OLLI KOSKIMIES

Nephrotic syndrome (NS) is characterized by proteinuria, low serum concentration of albumin and total proteins, hyperlipidemia and oedema. In case of prolonged nephrotic state disturbances of fluid and electrolyte balance, calcium depletion, thrombotic complications, bacterial infections and failure to thrive can create serious complications of the disease. The traditional classification is to divide NS to congenital, early infantile (symptoms detected before 3 months of age), infantile or childhood forms. In addition primary form imply a disease, in which the defect is located in the glomerular filter of the kidney itself, and secondary form on the other hand a generalized disease, for example a vasculitis, that also affects the kidney filtration barrier. It has to be pointed out, that the diagnosis has to be based on clinical (including the age of onset), laboratory, pathologic and genetic criteria. Especially the recent breakthroughs in the genetic nephrotic disorders will certainly give new insight to the development of proteinuria in the near future. NS presenting at birth or during early infancy is an uncommon disorder. Only about 6% of the total childhood nephrotic cohort are diagnosed under 1 year of age.